This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Idiopathic pulmonary fibrosis (IPF), also known as cryptogenic fibrosing alveolitis, is a distinct clinical disorder belonging to the spectrum of interstitial lung disease. IPF is a progressive disease characterized by the presence of a histological pattern of usual interstitial pneumonia (UIP) on surgical lung biopsy. No therapies have been shown to improve the survival or quality of life for patients with IPF. Current treatment is still based on the former presumption that IPF is an inflammatory process with concurrent remodeling of the lung by fibrosis. Consequently, it involves anti-inflammatory therapy, including corticosteroids (e.g., prednisone, prednisolone), immunosuppressive/cytotoxic agents (e.g., azathioprine, cyclophosphamide) or a combination of both. However, because of the marginal benefit and serious side effects of the current therapies, along with newer insights into the pathogenesis of IPF, novel therapeutic approaches are highly needed. Antifibrotic therapy is aimed at decreasing matrix deposition or increasing collagen breakdown and a number of agents including colchicine, D-penicillamine, interferon y, and pirfenidone, are currently under investigation. Lung transplantation has emerged as a viable option for some patients with IPF. Clinical and experimental studies suggest that bosentan would be safe and well tolerated and could delay the progression of IPF, a condition for which no established efficacious treatment is availale. The present BUILD-1 study investigates a posssible extension of the indication range of bosentan, which is currently approved for the treatment of moderate to severe PAH, to a new category of patients suffering from IPF.
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